Clinical Reviews in Allergy & Immunology

, Volume 28, Issue 2, pp 105–114

Overlap syndromes of cholestatic liver diseases and auto-immune hepatitis


DOI: 10.1385/CRIAI:28:2:105

Cite this article as:
Schramm, C. & Lohse, A.W. Clinic Rev Allerg Immunol (2005) 28: 105. doi:10.1385/CRIAI:28:2:105


Approximately 18% of patients with auto-immune liver disease present with features characteristic of a second auto-immune liver disease. These cases have been termed “overlap syndromes.” The pathogenesis of overlap syndromes is poorly understood, and few data are available regarding the clinical characteristics and outcome of this disease. Therefore, a consensus on the definition of overlap syndromes has not been reached. A common genetic background between auto-immune hepatitis (AIH) and its overlap with primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC) might confer susceptibility to a more inflammatory phenotype, probably requiring combined immunosuppressive treatment. This article focuses on the prevalence, diagnosis, and treatment of the overlap syndrome of AIH and PBC or PSC.

Index Entries

Auto-antibodyimmunogeneticsbiliary epithelial cellsT-cell receptorlymphocytes

Copyright information

© Human Press Inc 2005

Authors and Affiliations

  1. 1.Medizinische Klinik 1Universitätsulinikum Hamburg-EppendorfHamburgGermany